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Case 25-2003: a Newborn Boy with Petechiae and Thrombocytopenia

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Case 25-2003: a Newborn Boy with Petechiae and Thrombocytopenia The new england journal of medicine case records of the massachusetts general hospital Founded by Richard C. Cabot Nancy Lee Harris, m.d., Editor Sally H. Ebeling, Assistant Editor William F. McNeely, m.d., Associate Editor Stacey M. Ellender, Assistant Editor Jo-Anne O. Shepard, m.d., Associate Editor Christine C. Peters, Assistant Editor Case 25-2003: A Newborn Boy with Petechiae and Thrombocytopenia John F. Modlin, M.D., P. Ellen Grant, M.D., Robert S. Makar, M.D., Ph.D., Drucilla J. Roberts, M.D., and Kalpathy S. Krishnamoorthy, M.D., M.B., B.S. presentation of case A newborn boy was admitted to a special-care nursery because of petechiae and throm- From the Departments of Pediatrics and bocytopenia. Medicine, Dartmouth–Hitchcock Medical Center and Dartmouth Medical School, The boy had been delivered at this hospital at 39 weeks’ gestation to a 32-year-old Lebanon, N.H. (J.F.M.); the Departments woman who had had premature rupture of the membranes 16 hours before delivery. of Radiology (P.E.G.), Pathology (R.S.M., A single dose of penicillin was administered to the mother before vacuum-assisted de- D.J.R.), and Pediatrics (K.S.K.), Massachu- setts General Hospital and Harvard Medi- livery. The Apgar score was 9 at one minute and at five minutes. A diffuse petechial rash, cal School. most prominent on the face and trunk, was noted at delivery, and the baby was transferred to a newborn nursery. N Engl J Med 2003;349:691-700. The mother had never smoked, and she had not consumed alcohol after learning of Copyright © 2003 Massachusetts Medical Society. the pregnancy. Prenatal serologic tests were negative for hepatitis B surface antigen and syphilis, positive for antibodies to rubella and for IgG antibody to cytomegalovirus (CMV; 63 arbitrary units), and equivocal for IgM antibody to CMV (1 arbitrary unit; <0.9 unit is a negative result, and >1.1 units a positive result). Ultrasonographic examination of the fetus at approximately 20 weeks’ gestation revealed a focus of echogenicity within the heart and a hyperechoic bowel. Amniocentesis revealed that the chromosomes and the level of alpha-fetoprotein were normal; the fetal cells were negative for mutations in the cystic fibrosis gene. The estimated risk of Down’s syndrome was 1 in 85. The mother did not have a history of febrile illness, genital lesions, or rash during the preg- nancy. The family history was unremarkable on both the maternal and the paternal sides. The infant’s temperature was 36.6°C, the pulse 110 beats per minute, and the respi- ratory rate 48 breaths per minute. The blood pressure was 60/40 mm Hg. The weight was 2.29 kg (below the 10th percentile), the length 46 cm (between the 10th and 25th percen- tiles), and the head circumference 33 cm (at the 25th percentile). On physical examination, the infant appeared well and comfortable; his features were not dysmorphic, and he was not in respiratory distress. Multiple petechial lesions were present on his face, trunk, and arms and legs and were especially numerous at the site on the head where the vacuum cup had been placed (Fig. 1). No lymphadenopathy was found. The anterior fontanelle was open and flat. The eyes showed no microphthal- mia, icterus, or cataracts; the retinas were not examined. The clavicles were intact, and the lungs were clear. The heart sounds were normal. The abdomen was soft; the spleen n engl j med 349;7 www.nejm.org august 14, 2003 691 The New England Journal of Medicine Downloaded from nejm.org at INSERM DISC DOC on November 10, 2011. For personal use only. No other uses without permission. Copyright © 2003 Massachusetts Medical Society. All rights reserved. The new england journal of medicine ing associated with infection with toxoplasma, CMV, togavirus (the agent that causes rubella), and herpes simplex virus and with increased risks of Down’s syndrome, cystic fibrosis, bleeding, and growth re- tardation. Dr. Stephen Walsh (Infectious Disease Unit): We were asked to see the infant the day he was born. There was a diffuse, macular, petechial eruption on his face but no pustules or vesicles (Fig. 1). Figure 1. Photograph of the Patient, Showing Petechiae on the Forehead. Dr. Modlin: This newborn infant had intrauter- ine growth retardation and shortly after birth was found to have hepatosplenomegaly, thrombocy- topenia, and hyperbilirubinemia. It is helpful to was palpable 2.5 to 3.0 cm below the left costal mar- begin by considering the broad categories of con- gin, and the liver edge 3.0 cm below the right costal ditions associated with thrombocytopenia in new- margin. The arms and legs were well perfused. borns (Table 2). Muscle tone was good throughout, with appropriate Maternal antiplatelet antibodies are an impor- Moro’s and sucking reflexes. tant cause of thrombocytopenia in newborns, but For several hours in the nursery, the infant’s their presence is not accompanied by hepatosple- heart rate was in the range of 100 to 110 beats per nomegaly or intrauterine growth retardation. There minute, with occasional drops to 85 beats per min- are no features of this case that suggest the pres- ute. The arterial oxygen saturation occasionally de- ence of structural birth defects such as absent radii clined from 95 percent or more to about 85 percent. or a large hemangioma (the Kasabach–Merritt syn- The results of laboratory tests are given in Table 1. drome), and the complete blood count is not con- The levels of urea nitrogen, creatinine, total protein, sistent with the presence of congenital leukemia. triglyceride, aspartate aminotransferase, and ala- There are several metabolic diseases and other ge- nine aminotransferase were normal. Examination netic disorders, such as Fanconi’s anemia and the of a peripheral-blood smear disclosed hypochro- Wiskott–Aldrich syndrome, that may be manifested mia (++), with macrocytosis, polychromasia (+), as neonatal thrombocytopenia, but usually not on and teardrop cells. Specimens of blood, cerebrospi- the first day of life. This quick process of elimina- nal fluid, and urine were obtained for culture, and tion leaves infection as the probable cause of the ampicillin, tobramycin, and acyclovir were given. infant’s thrombocytopenia. Early-onset bacterial Breast-feeding was begun. The next day, the oxygen sepsis was appropriately considered in this case, saturation ranged from 93 to 98 percent while the and the infant underwent a workup for sepsis and patient was breathing ambient air. received broad-spectrum antibiotics. However, there A diagnostic procedure was performed. were no risk factors for neonatal sepsis, such as ma- ternal fever, chorioamnionitis, or premature labor. differential diagnosis The normal white-cell count and the relatively be- nign course of illness make this diagnosis even less Dr. John F. Modlin: May we see the prenatal radiolog- likely. ic studies and the clinical photographs? The main clinical features in this case are most Dr. Susan A. Connolly (Radiology): An intracardi- consistent with an infection acquired in utero. Ta- ac focus of echogenicity, which represents a small ble 3 lists the organisms causing intrauterine infec- amount of calcification, usually in the papillary mus- tions that might have some or all of the principal cle, was visible on a cross-section image of the fetal features seen in this case — namely, thrombocyto- chest showing the oblique four-chamber view of penia, hepatosplenomegaly, and intrauterine growth the heart. This finding can be a normal variant; it is retardation.1-6 Table 3 also includes my best esti- seen in 5 percent of normal fetuses on second-tri- mate of the current overall incidence of each of these mester scans. However, it has been associated with infections and the likelihood that a child will have an approximately doubled risk of Down’s syndrome. symptoms and signs at birth. CMV is the most com- The hyperechoic bowel, which was best seen on an mon agent in the differential diagnosis and thus oblique transverse image of the abdomen, is a find- must be considered in any infant who is thought to 692 n engl j med 349;7 www.nejm.org august 14, 2003 The New England Journal of Medicine Downloaded from nejm.org at INSERM DISC DOC on November 10, 2011. For personal use only. No other uses without permission. Copyright © 2003 Massachusetts Medical Society. All rights reserved. case records of the massachusetts general hospital have an intrauterine infection. Congenital toxo- plasmosis is the infection most likely to be confused Table 1. Laboratory Data.* with CMV infection, but population-based screen- Variable 2 Hours 6 Hours Day 2 ing in the United States indicates that toxoplas- 3 mosis infects newborns much less often than CMV White-cell count (per mm ) 13,300 18,300 19,100 infection. There are also distinguishing clinical fea- Hematocrit (%) 54.8 59.6 52.3 tures; the rash observed in infants with congenital Hemoglobin (g/dl) 18.2 19.2 17.6 toxoplasmosis is usually maculopapular rather than Red-cell count (per mm3) 5,530 5,900 5,240 petechial, and infants with toxoplasmosis have cho- 3 rioretinitis more often than those with congenital Platelet count (per mm ) 50,000 57,000 68,000 CMV infection. Mean corpuscular volume (µm3) 99 101 100 Vertically transmitted infection with the human Mean corpuscular hemoglobin 32.9 32.5 33.5 immunodeficiency virus (HIV) has dropped from (pg/red cell) a peak of approximately 1600 cases in 1992 to fewer Mean corpuscular hemoglobin 33.1 32.2 33.6 than 200 cases in 2000. In the United States, approx- concentration (g/dl) imately 30 percent of HIV-infected infants who are Red-cell–distribution width (%) 19.5 19.2 19.6 not being breast-fed acquired the infection during Differential count (%) gestation, but they usually have no symptoms at birth.
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